[1]
Athanazio RA,Silva Filho LVRF,Vergara AA,Ribeiro AF,Riedi CA,Procianoy EDFA,Adde FV,Reis FJC,Ribeiro JD,Torres LA,Fuccio MB,Epifanio M,Firmida MC,Damaceno N,Ludwig-Neto N,Maróstica PJC,Rached SZ,Melo SFO, Brazilian guidelines for the diagnosis and treatment of cystic fibrosis. Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia. 2017 May-Jun;
[PubMed PMID: 28746534]
[2]
Boëlle PY,Debray D,Guillot L,Clement A,Corvol H, Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients. Hepatology (Baltimore, Md.). 2019 Apr;
[PubMed PMID: 30058245]
[4]
Paranjape SM,Mogayzel PJ Jr, Cystic fibrosis in the era of precision medicine. Paediatric respiratory reviews. 2018 Jan;
[PubMed PMID: 28372929]
[5]
Keogh RH,Szczesniak R,Taylor-Robinson D,Bilton D, Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2018 Mar;
[PubMed PMID: 29311001]
[6]
Debray D,Kelly D,Houwen R,Strandvik B,Colombo C, Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2011 Jun;
[PubMed PMID: 21658639]
[7]
Zsembery A,Jessner W,Sitter G,Spirlí C,Strazzabosco M,Graf J, Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells. Hepatology (Baltimore, Md.). 2002 Jan;
[PubMed PMID: 11786964]
[8]
Fiorotto R,Strazzabosco M, Cystic Fibrosis-Related Liver Diseases: New Paradigm for Treatment Based on Pathophysiology. Clinical liver disease. 2016 Nov;
[PubMed PMID: 31041076]
[9]
Colombo C,Battezzati PM,Crosignani A,Morabito A,Costantini D,Padoan R,Giunta A, Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology (Baltimore, Md.). 2002 Dec;
[PubMed PMID: 12447862]
[10]
Narkewicz MR, Markers of cystic fibrosis-associated liver disease. Journal of pediatric gastroenterology and nutrition. 2001 Apr;
[PubMed PMID: 11396806]
[11]
Hamosh A,FitzSimmons SC,Macek M Jr,Knowles MR,Rosenstein BJ,Cutting GR, Comparison of the clinical manifestations of cystic fibrosis in black and white patients. The Journal of pediatrics. 1998 Feb;
[PubMed PMID: 9506637]
[12]
Debray D,Lykavieris P,Gauthier F,Dousset B,Sardet A,Munck A,Laselve H,Bernard O, Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension. Journal of hepatology. 1999 Jul;
[PubMed PMID: 10424286]
[13]
Fiorotto R,Strazzabosco M, Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota. Cellular and molecular gastroenterology and hepatology. 2019;
[PubMed PMID: 31075352]
[14]
Witters P,Libbrecht L,Roskams T,De Boeck K,Dupont L,Proesmans M,Vermeulen F,Maleux G,Monbaliu D,Pirenne J,Cassiman D, Liver disease in cystic fibrosis presents as non-cirrhotic portal hypertension. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2017 Sep;
[PubMed PMID: 28347603]
[15]
Lewindon PJ,Pereira TN,Hoskins AC,Bridle KR,Williamson RM,Shepherd RW,Ramm GA, The role of hepatic stellate cells and transforming growth factor-beta(1) in cystic fibrosis liver disease. The American journal of pathology. 2002 May;
[PubMed PMID: 12000722]
[16]
Mueller-Abt PR,Frawley KJ,Greer RM,Lewindon PJ, Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2008 May;
[PubMed PMID: 17904429]
[17]
Kobelska-Dubiel N,Klincewicz B,Cichy W, Liver disease in cystic fibrosis. Przeglad gastroenterologiczny. 2014;
[PubMed PMID: 25097709]
[18]
Chapman R,Fevery J,Kalloo A,Nagorney DM,Boberg KM,Shneider B,Gores GJ, Diagnosis and management of primary sclerosing cholangitis. Hepatology (Baltimore, Md.). 2010 Feb;
[PubMed PMID: 20101749]
[19]
Diwakar V,Pearson L,Beath S, Liver disease in children with cystic fibrosis. Paediatric respiratory reviews. 2001 Dec;
[PubMed PMID: 12052306]
[21]
Lenaerts C,Lapierre C,Patriquin H,Bureau N,Lepage G,Harel F,Marcotte J,Roy CC, Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors. The Journal of pediatrics. 2003 Sep;
[PubMed PMID: 14517517]
[22]
Witters P,De Boeck K,Dupont L,Proesmans M,Vermeulen F,Servaes R,Verslype C,Laleman W,Nevens F,Hoffman I,Cassiman D, Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2009 Dec;
[PubMed PMID: 19733131]
[23]
Sadler MD,Crotty P,Fatovich L,Wilson S,Rabin HR,Myers RP, Noninvasive methods, including transient elastography, for the detection of liver disease in adults with cystic fibrosis. Canadian journal of gastroenterology
[PubMed PMID: 25855877]
[24]
Calvopina DA,Noble C,Weis A,Hartel GF,Ramm LE,Balouch F,Fernandez-Rojo MA,Coleman MA,Lewindon PJ,Ramm GA, Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2019 Jul 11;
[PubMed PMID: 31303380]
[25]
Lewindon PJ,Shepherd RW,Walsh MJ,Greer RM,Williamson R,Pereira TN,Frawley K,Bell SC,Smith JL,Ramm GA, Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy. Hepatology (Baltimore, Md.). 2011 Jan;
[PubMed PMID: 21254170]
[26]
Koh C,Sakiani S,Surana P,Zhao X,Eccleston J,Kleiner DE,Herion D,Liang TJ,Hoofnagle JH,Chernick M,Heller T, Adult-onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity. Hepatology (Baltimore, Md.). 2017 Aug;
[PubMed PMID: 28422310]
[27]
Palaniappan SK,Than NN,Thein AW,Moe S,van Mourik I, Interventions for preventing and managing advanced liver disease in cystic fibrosis. The Cochrane database of systematic reviews. 2017 Aug 29;
[PubMed PMID: 28850173]
Level 1 (high-level) evidence
[28]
Flass T,Narkewicz MR, Cirrhosis and other liver disease in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2013 Mar;
[PubMed PMID: 23266093]
[29]
Funakoshi N,Duny Y,Valats JC,Ségalas-Largey F,Flori N,Bismuth M,Daurès JP,Blanc P, Meta-analysis: beta-blockers versus banding ligation for primary prophylaxis of esophageal variceal bleeding. Annals of hepatology. 2012 May-Jun;
[PubMed PMID: 22481457]
Level 1 (high-level) evidence
[30]
Colombo C,Crosignani A,Assaisso M,Battezzati PM,Podda M,Giunta A,Zimmer-Nechemias L,Setchell KD, Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. Hepatology (Baltimore, Md.). 1992 Oct;
[PubMed PMID: 1398498]
[31]
Lamireau T,Monnereau S,Martin S,Marcotte JE,Winnock M,Alvarez F, Epidemiology of liver disease in cystic fibrosis: a longitudinal study. Journal of hepatology. 2004 Dec;
[PubMed PMID: 15582124]
[32]
Staufer K,Halilbasic E,Trauner M,Kazemi-Shirazi L, Cystic fibrosis related liver disease--another black box in hepatology. International journal of molecular sciences. 2014 Aug 4;
[PubMed PMID: 25093717]
[33]
Barry PJ,Plant BJ,Nair A,Bicknell S,Simmonds NJ,Bell NJ,Shafi NT,Daniels T,Shelmerdine S,Felton I,Gunaratnam C,Jones AM,Horsley AR, Effects of ivacaftor in patients with cystic fibrosis who carry the G551D mutation and have severe lung disease. Chest. 2014 Jul;
[PubMed PMID: 24522694]
[34]
Wark PAB,Cookson K,Thiruchelvam T,Brannan J,Dorahy DJ, Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction. BMC pulmonary medicine. 2019 Jun 17;
[PubMed PMID: 31208380]
[35]
Freeman AJ,Sellers ZM,Mazariegos G,Kelly A,Saiman L,Mallory G,Ling SC,Narkewicz MR,Leung DH, A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis-Associated Liver Disease. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. 2019 Apr;
[PubMed PMID: 30697907]
[36]
Colombo C,Costantini D,Rocchi A,Romano G,Rossi G,Bianchi ML,Bertoli S,Battezzati A, Effects of liver transplantation on the nutritional status of patients with cystic fibrosis. Transplant international : official journal of the European Society for Organ Transplantation. 2005 Feb;
[PubMed PMID: 15691279]
[37]
Lu BR,Esquivel CO, A review of abdominal organ transplantation in cystic fibrosis. Pediatric transplantation. 2010 Dec;
[PubMed PMID: 20946451]
[38]
Gridelli B, Liver: Benefit of liver transplantation in patients with cystic fibrosis. Nature reviews. Gastroenterology
[PubMed PMID: 21386811]
[39]
Miller MR,Sokol RJ,Narkewicz MR,Sontag MK, Pulmonary function in individuals who underwent liver transplantation: from the US cystic fibrosis foundation registry. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. 2012 May;
[PubMed PMID: 22271602]
[40]
Abdalian R,Heathcote EJ, Sclerosing cholangitis: a focus on secondary causes. Hepatology (Baltimore, Md.). 2006 Nov;
[PubMed PMID: 17058222]
[41]
Jong T,Geake J,Yerkovich S,Bell SC, Idiosyncratic reactions are the most common cause of abnormal liver function tests in patients with cystic fibrosis. Internal medicine journal. 2015 Apr;
[PubMed PMID: 25644776]
[42]
Temple JL,Cordero P,Li J,Nguyen V,Oben JA, A Guide to Non-Alcoholic Fatty Liver Disease in Childhood and Adolescence. International journal of molecular sciences. 2016 Jun 15;
[PubMed PMID: 27314342]
[43]
Feranchak AP,Sokol RJ, Cholangiocyte biology and cystic fibrosis liver disease. Seminars in liver disease. 2001 Nov;
[PubMed PMID: 11745036]
[44]
Ayoub F,Trillo-Alvarez C,Morelli G,Lascano J, Risk factors for hepatic steatosis in adults with cystic fibrosis: Similarities to non-alcoholic fatty liver disease. World journal of hepatology. 2018 Jan 27;
[PubMed PMID: 29399276]
[45]
FitzSimmons SC, The changing epidemiology of cystic fibrosis. Current problems in pediatrics. 1994 May-Jun;
[PubMed PMID: 8070278]
[46]
Colombo C,Apostolo MG,Ferrari M,Seia M,Genoni S,Giunta A,Sereni LP, Analysis of risk factors for the development of liver disease associated with cystic fibrosis. The Journal of pediatrics. 1994 Mar;
[PubMed PMID: 8120708]
[47]
Lindblad A,Glaumann H,Strandvik B, Natural history of liver disease in cystic fibrosis. Hepatology (Baltimore, Md.). 1999 Nov;
[PubMed PMID: 10534335]
[48]
Gooding I,Dondos V,Gyi KM,Hodson M,Westaby D, Variceal hemorrhage and cystic fibrosis: outcomes and implications for liver transplantation. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. 2005 Dec;
[PubMed PMID: 16258952]